回顾性分析 2015 年 3 月至 2016 年 8 月在本院收治的先天性心脏病合并肺动脉高压 60 例患儿的临床资料。其中 30 例患儿合并唐氏综合征为试验组，另外 30 例患儿为对照组。两组患儿均行择期手术治疗，并于术后给予法舒地尔联合西地那非治疗肺动脉高压。
两组患儿术中体外循环时间、主动脉阻断时间、改良超滤时间和术后呼吸道并发症发生率无显著差异。与术前相比，两组患儿术后 24 h 肺动脉收缩压均有显著下降（P 均<0.05）。与对照组相比，试验组患儿术后 6 h 氧分压和氧合指数均显著降低（P 均<0.05）。试验组患儿术后机械通气时间和重症监护时间较对照组延长（分别为P=0.007 和 P=0.000）。无近期死亡和再手术患者。
To explore the treatment method of congenital heart disease (CHD) with pulmonary artery hypertension (PAH) in infants with Down syndrome (DS).
Sixty CHD patients with PAH from March 2015 to August 2016 were retrospectively analysed. There were 30 infants were diagnosed as a DS group (trial group) and the other 30 patients were classified as a control group. All the patients underwent surgical treatment and fasudil combined with sildenafil were used to prevent pulmonary hypertension crisis postoperatively.
There were no significant differences in cardiopulmonary bypass time, aortic cross-clamping time, modified ultrafiltration time and the incidence of postoperative respiratory complications between the two groups. The pulmonary systolic blood pressure significantly decreased at 24 h after operation (both P<0.05). The arterial oxygen pressure and oxygenation index of the trial group were lower than that of the control group at 6 h after operation (bothP<0.05). The mechanical ventilation time and intensive care time of the trial group were significantly longer than those of the control group (P=0.007 and P=0.000, respectively). There were no reoperations or early death.
The effects of surgical repair of congenital heart disease with PAH in infants with DS were satisfactory by grasping the indication, protecting lung function and controlling of PAH in the early postoperative period, although there is a high incidence of pulmonary complication.
congenital heart disease;
pulmonary artery hypertension